title: Prevalence of dural ectasia in Loeys-Dietz syndrome: comparison with Marfan syndrome and normal controls.
abstract: OBJECTIVE
  Dural ectasia is well recognized in Marfan syndrome (MFS) as one of the major diagnostic criteria, but the exact prevalence of dural ectasia is still unknown in Loeys-Dietz syndrome (LDS), which is a recently discovered connective tissue disease. In this study, we evaluated the prevalence of dural ectasia in LDS according by using qualitative and quantitative methods and compared our findings with those for with MFS and normal controls.
  
  
  METHODS
  We retrospectively studied 10 LDS (6 males, 4 females, mean age 36.3 years) and 20 MFS cases (12 males, 8 females, mean age 37.1 years) and 20 controls (12 males, 8 females, mean age 36.1 years) both qualitatively and quantitatively using axial CT images and sagittal multi-planar reconstruction images of the lumbosacral region. For quantitative examination, we adopted two methods: method-1 (anteroposterior dural diameter of S1> L4) and method-2 (ratio of anteroposterior dural diameter/vertebral body diameter>cutoff values). The prevalence of dural ectasia among groups was compared by using Fisher's exact test and the Tukey-Kramer test.
  
  
  RESULTS
  In LDS patients, the qualitative method showed 40% of dural ectasia, the quantitative method-1 50%, and the method-2 70%. In MFS patients, the corresponding prevalences were 50%, 75%, and 85%, and in controls, 0%, 0%, and 5%. Both LDS and MFS had a significantly wider dura than controls.
  
  
  CONCLUSIONS
  While the prevalence of dural ectasia varied depending on differences in qualitative and quantitative methods, LDS as well as MFS, showed, regardless of method, a higher prevalence of dural ectasia than controls. This finding should help the differentiation of LDS from controls.
authors.affiliation: Department of Radiology, Kobe University Graduate School of Medicine, Kobe Japan.
authors.forename: Atsushi K
authors.initials: AK
authors.lastname: Kono
authors.forename: Masahiro
authors.initials: M
authors.lastname: Higashi
authors.forename: Hiroko
authors.initials: H
authors.lastname: Morisaki
authors.forename: Takayuki
authors.initials: T
authors.lastname: Morisaki
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Naito
authors.forename: Kazuro
authors.initials: K
authors.lastname: Sugimura
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D004108:Dilatation, Pathologic / Q000453:epidemiology* / Q000473:pathology*; D004388:Dura Mater / Q000473:pathology*; D005260:Female; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology*; D008161:Lumbosacral Region / Q000000981:diagnostic imaging*; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis / Q000473:pathology; D008875:Middle Aged; D015995:Prevalence; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed